title: Phaeochromocytoma in black South Africans - a 30-year audit.
abstract: OBJECTIVE
  Phaeochromocytomas are catecholamine-secreting tumours, the majority of which arise from the adrenal medulla. Untreated, they are potentially lethal; early diagnosis and treatment offer a good chance of cure. They are rarely reported in blacks. The clinical presentation and outcome of phaeochromocytoma in a large cohort of black South Africans is reviewed.
  
  
  METHODS
  Patients' records in a tertiary care university hospital were reviewed. Fifty-four black patients presenting with phaeochromocytoma between 1980 and 2009 were included. The clinical presenting features, tumour localisation and outcome were assessed.
  
  
  RESULTS
  Fifty-four (41 female, 13 male; age range 8 - 57 years) patients were identified. Five (9%) had familial syndromes; 49 (91%) were deemed sporadic. All tumours were intra-abdominal: 34 (61%) were adrenal and 22 (39%) extra-adrenal in origin. The most common symptoms were headache (77%), palpitations (77%), and sweating (74%). All were hypertensive, almost equally divided between paroxysmal and sustained hypertension. Six (11%) presented in congestive cardiac failure including 2 with catecholamine-induced myocarditis. Two patients had features which simulated hypertrophic obstructive cardiomyopathy. Nine women presented in pregnancy: there was no maternal mortality; fetal mortality included 1 early neonatal death and 1 intrauterine death. There were 4 deaths: 1 from postoperative haemorrhage, 1 from multisystem crisis, 1 from metastatic medullary thyroid carcinoma, and 1 from catecholamine-induced myocarditis.
  
  
  CONCLUSIONS
  Phaeochromocytoma is an important although rare tumour in blacks, with similar clinical presentations and complications to those in white patients. Timely diagnosis and appropriate treatment resulted in a favourable outcome in over 90% of patients in this study.
authors.affiliation: University of Witwatersrand, Johannesburg, South Africa. kenneth.huddle@wits.ac.za
authors.forename: K R L
authors.initials: KR
authors.lastname: Huddle
mesh_terms: D000293:Adolescent; D000310:Adrenal Gland Neoplasms / Q000453:epidemiology* / Q000235:genetics; D000328:Adult; D017677:Age Distribution; D044383:Black People; D002648:Child; D005260:Female; D020022:Genetic Predisposition to Disease; D006331:Heart Diseases / Q000453:epidemiology / Q000209:etiology; D006801:Humans; D006973:Hypertension / Q000453:epidemiology / Q000209:etiology; D008297:Male; D008485:Medical Audit; D008875:Middle Aged; D010673:Pheochromocytoma / Q000453:epidemiology* / Q000235:genetics; D011247:Pregnancy; D011252:Pregnancy Complications, Neoplastic / Q000453:epidemiology; D051096:Proto-Oncogene Proteins c-ret / Q000235:genetics; D017678:Sex Distribution; D013019:South Africa / Q000453:epidemiology; D051794:Von Hippel-Lindau Tumor Suppressor Protein / Q000235:genetics; D055815:Young Adult
chemical_list: D051794:Von Hippel-Lindau Tumor Suppressor Protein; D051096:Proto-Oncogene Proteins c-ret; C497446:VHL protein, human